ARTICLE
A premature, 1-month-old infant, with neonatal enterocolitis and receiving
parenteral nutrition, developed an erosive, erythematous rash, with multiple
blisters spreading to the face (Fig.
1), genital region and thighs (Fig.
2). In addition to the rash, the baby had a fever and was irritable.
The baby's white blood cell count was 15,800/mm3, and the C-reactive
protein level was 85 g/dl. Bacterial culture of the blood isolated Staphylococcus
aureus which was methicilline resistant, and a mycologic culture of
urine and stool isolated Candida parapsilosis. Bacterial cultures
of the central and umbilical catheter, the throat and gastric liquid were
negative. A skin biopsy was taken and histological examination revealed
epidermal hyperkeratosis with parakeratosis and a dermal infiltrate of
mononuclear cells (Fig. 3).
Direct immunofluorescence of the skin biopsy was negative. The rash failed
to respond to topical antibacterial cream (fusidic acid) and systemic
antibacterial treatments (vancomycin, amikacin). The antifungal fluconazole
was added intravenously and oral nutritional supplements were begun. What
is your diagnosis?
Acrodermatitis enteropathica-like
rash and enterocolitis
We diagnosed acquired acrodermatitis enteropathica developing during
parenteral nutrition for enterocolitis. The erythematous, desquamating
rash in the perioral area and affecting the genital and pressure areas
as well as the thighs led to the diagnosis of acrodermatitis enteropathica.
Danbolt and Closs [1] coined the name ''acrodermatitis enteropathica''
(AE) in 1942 to describe a rash, comprising vesicles, bullae or erosions,
primarily located acrally, in some infants with diarrhea and alopecia.
Moynaham [2] in 1974, discovered that AE, inherited in some cases in an
autosomal recessive manner, was associated with zinc deficiency. Zinc
deficiency can be caused by intravenous hypernutrition, accelerated tissue
metabolism due to infection, protein energy malnutrition, gastrointestinal
disease, low dietary intake or chemotherapy [3]. Today, some studies attempting
to elucidate the precise mechanisms of zinc deficiency, have suggested
impaired zinc absorption due to the lack of a certain zinc-binding ligand
or an impairment in zinc metabolism. Hansen et al. [4] in 1983,
described a refractory, AE-like eruption similar in some respects to that
of AE. They observed a rash that had begun with erythematous papules that
had then expanded to become plaques, which coalesced and desquamated.
Histopathological findings in both AE and this AE-like rash were nonspecific
with parakeratosis. An AE rash responds quickly to nutritional supplement,
but most infants also need additional zinc supplementation [5]. The infant
described here had a low serum zinc level (5.8 µmol/l ; 10.7 to 16.8
µmol/l is the normal range). The baby had
not been given zinc supplementation, but because of infection, the central
catheter was removed and oral nutrition given. The AE-like rash in this
child was probably caused by zinc deficiency, as reflected in the child's
low serum zinc level. But other causes can be considered in this diagnosis,
such as biotin or amino acid deficiency, lipid abnormalities, as all these
deficiencies can be observed in patients with enterocolitis and those
receiving parenteral nutrition. In addition to AE, this rash bears some
similarities to rashes observed in essential fatty acid deficiencies,
glucagonoma syndrome, isoleucine deficiency, severe seborrheic dermatitis,
and psoriasis.
Dietary supplementation was successful and the
rash resolved within four days which further supports our diagnosis that
this rash was due to a zinc deficiency. The child is today completely
symptom-free. Children receiving nutrition intravenously or suffering
from infection are at particular risk of zinc deficiency and therefore
need to be monitered and given zinc supplementation when necessary.
REFERENCES
1. Danbolt N, Closs K. Acrodermatitis enteropathica. Acta Derm Venereol
1942; 23: 127-69.
2. Moynahan EJ. Acrodermatitis enteropathica: a lethal inherited human
zinc-deficiency disorder. Lancet 1974; 2: 399-400.
3. Sawai T, Sugiura H, Danno K, Uchiyama M, Ohta S. Acquired acrodermatitis
enteropathica during chemotherapy for acute lymphocytic leukaemia in a
child with Down's syndrome. Br J Dermatol 1996; 135: 652-68.
4. Hansen RC, Lemen R, Revsin B. Cystic fibrosis manifesting with acrodermatitis
enteropathica-like eruption: association with essential fatty acid and
zinc deficiencies. Arch Dermatol 1983; 119: 51.
5. Ghali FE, Steinberg JB, Tunnessen WW. Picture of the month. Arch
Pediatr Adolesc Med 1996; 150: 99-100.
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